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INTRODUCTION: Few studies have longitudinally mapped quality of life (QoL) trajectories of newly diagnosed people with dementia and their carers, particularly during coronavirus disease-2019 (COVID-19). METHODS: In a UK cohort study, 261 newly diagnosed people with dementia and 206 family carers were assessed prior to the pandemic (July 2019-March 2020), followed up after the first lockdown (July-October 2020) and then again a year and 2 years later. Latent growth curve modelling examined the level and change of QoL over the four time-points using dementia-specific QoL measures (DEMQOL and C-DEMQOL). RESULTS: Despite variations in individual change scores, our results suggest that generally people with dementia maintained their QoL during the pandemic and experienced some increase towards the end of the period. This contrasted with carers who reported a general deterioration in their QoL over the same period. 'Confidence in future' and 'Feeling supported' were the only carer QoL subscales to show some recovery post-pandemic. DISCUSSION: It is positive that even during a period of global disruption, decline in QoL is not inevitable following the onset of dementia. However, it is of concern that carer QoL declined during this same period even after COVID-19 restrictions had been lifted. Carers play an invaluable role in the lives of people with dementia and wider society, and our findings suggest that, post-pandemic, they may require greater support to maintain their QoL.
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COVID-19 , Demência , Humanos , Qualidade de Vida , Cuidadores , Demência/epidemiologia , Demência/diagnóstico , Pandemias , Estudos de Coortes , COVID-19/epidemiologia , Controle de Doenças TransmissíveisRESUMO
OBJECTIVE: Perampanel (PER) has been shown to be effective as an adjunctive therapy for controlling refractory focal-onset seizures (FOS). However, the information as early add-on for the treatment of FOS in the clinical practice is still scarce and must be further assessed. METHODS: An observational prospective study was conducted to evaluate the effectiveness of early add-on PER, assessed as 50% responders (seizure frequency reduced by at least 50% during the last 3â¯months as compared with baseline) rate at 6 and 12â¯months, in patients with FOS in the routine clinical practice of Spain. RESULTS: One hundred and thirteen patients (mean age: 40.3â¯years, 51.3% male) with FOS received PER as early add-on (1st add-on: 37.2% and 2nd: 62.8%) for a mean exposure of 11â¯months (mean PER dose: 6.3â¯mg/day at month 12). At 6â¯months, 50.4% and 20.4% of the patients were responders and seizure-free (respectively) relative to baseline (3â¯months prior to PER initiation), and at 12â¯months, 68.1% and 26.5% of the patients were responders and seizure-free (respectively), relative to baseline (3â¯months prior to PER initiation). The retention rate at 6 and 12â¯months was 83.2% and 80.5%, respectively. The percentage of seizure-free patients at 12â¯months was significantly (pâ¯=â¯0.033) higher when PER was added as first vs. second add-on. The number of concomitant antiepileptic drugs (AEDs) was significantly reduced from baseline to 6 and 12â¯months (pâ¯=â¯0.001). Treatment was simplified in 23.9% of patients at the end of the observation period. Drug-related adverse events (AEs), most mild or moderate, were reported in 30.1% of patients, with irritability (8%) and dizziness (7.1%) as the most frequent ones. CONCLUSIONS: This is the first observational, prospective study to evaluate efficacy and safety of early adjunctive treatment with PER in patients with focal epilepsy at 12â¯months. Perampanel demonstrated a good efficacy and safety profile when used at a median dose of 6â¯mg/day, regardless of the combination with other AEDs. Adverse events were mild or moderate, with dizziness being the most frequent one.
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Anticonvulsivantes/administração & dosagem , Epilepsia/tratamento farmacológico , Epilepsia/epidemiologia , Piridonas/administração & dosagem , Convulsões/tratamento farmacológico , Convulsões/epidemiologia , Adulto , Anticonvulsivantes/efeitos adversos , Tontura/induzido quimicamente , Quimioterapia Combinada , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Nitrilas , Estudos Prospectivos , Piridonas/efeitos adversos , Espanha/epidemiologia , Resultado do Tratamento , Adulto JovemRESUMO
Introduction: Interest in 3D printing for orthopedic surgery has been increasing since its progressive adoption in most of the hospitals around the world. The aim of the study is to describe all the current applications of 3D printing in patients undergoing hip surgery of any type at the present time. Materials and Methods: We conducted a systematic narrative review of publications indexed in MedLine through the search engine PubMed, with the following parameters: 3D printing AND (orthopedics OR traumatology) NOT tissue engineering NOT scaffold NOT in vitro and deadline 31 July 2023. After reading the abstracts of the articles, papers were selected according to the following criteria: full text in English or Spanish and content related to hip surgery. Those publications involving experimental studies (in vitro or with anatomical specimens) or 3D printing outside of hospital facilities as well as 3D-printed commercial implants were excluded. Results are presented as a reference guide classified by disease, including the used software and the steps required for the development of the idea. Results: We found a total of 27 indications for in-house 3D printing for hip surgery, which are described in the article. Conclusions: There are many surgical applications of 3D printing in hip surgery, most of them based on CT images. Most of the publications lack evidence, and further randomized studies should be encouraged to assess the advantages of these indications.
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We evaluated predictors of the Clinical Frailty Scale (CFS) scored by an interdisciplinary team (Home FIRsT) performing comprehensive geriatric assessment (CGA) in our Emergency Department (ED). This was a retrospective observational study (service evaluation) utilising ED-based CGA data routinely collected by Home FIRsT between January and October 2020. A linear regression model was computed to establish independent predictors of CFS. This was complemented by a classification and regression tree (CRT) to evaluate the main predictors. There were 799 Home FIRsT episodes, of which 740 were unique patients. The CFS was scored on 658 (89%) (median 4, range 1-8; mean age 81 years, 61% women). Independent predictors of higher CFS were older age (p<0.001), history of dementia (p<0.001), mobility (p≤0.007), disability (p<0.001), and higher acuity of illness (p=0.009). Disability and mobility were the main classifiers in the CRT. Results suggest appropriate CFS scoring informed by functional baseline.
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An interesting evolutionary question that still remains open is the connectivity between marine populations. Marine currents can favour the dispersal of larvae or adults, but they can also produce eddies and gyres generating oceanographic fronts, thus limiting gene flow. To address this subject, we selected the Atlantic-Mediterranean transition, where several fronts are located: Gibraltar Strait (GS), Almeria-Oran Front (AOF) and Ibiza Channel (IC). Seven populations of the marine crab Liocarcinus depurator (Cadiz, West and East Alboran, Alacant, Valencia, Ebro Delta and North Catalonia) located along this transition were analysed in six consecutive years (2014-2019) using a fragment of the COI (Cytochrome Oxidase subunit I) gene. All sequences (966) belonged to two well defined haplogroups: ATL (most abundant in Atlantic waters) and MED (predominant in Mediterranean waters). Following a geographic variation, the frequency of ATL decreased significantly from Cadiz to North Catalonia. However, this variation presented steps due to the effect of oceanographic restrictions/fronts. Significant effects were recorded for GS (2015, 2017, 2018 and 2019), AOF (all years except 2018) and IC (2016). The intensity and precise location of these fronts changed over time. Multivariate analyses distinguished three main population groups: Cadiz, Alboran Sea and the remaining Mediterranean populations. These findings could be relevant to properly define Marine Protected Areas and for conservation and fisheries policies.
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Braquiúros/fisiologia , Animais , Oceano Atlântico , Evolução Biológica , Braquiúros/genética , Conservação dos Recursos Naturais , Complexo IV da Cadeia de Transporte de Elétrons/genética , Política Ambiental , Pesqueiros/normas , Fluxo Gênico , Variação Genética , Humanos , Mar Mediterrâneo , Formulação de Políticas , Movimentos da ÁguaRESUMO
Megalopas of 15 brachyuran crab species collected in the open sea plankton, and unknown until now, were identified using DNA barcodes (COI and 16S rRNA). Specimens belonging to the families Portunidae, Pseudorhombilidae and Xanthidae (Crustacea, Decapoda, Brachyura), and corresponding to the species Achelous floridanus, Arenaeus mexicanus, Callinectes amnicola, C. arcuatus, C. ornatus, C. toxones, Charybdis (Charybdis) hellerii, Portunus hastatus, Thalamita admete, Scopolius nuttingi, Etisus odhneri, Liomera cinctimanus, Neoliomera cerasinus, Pseudoliomera variolosa, and Williamstimpsonia stimpsoni, are described and illustrated, and compared with other congeneric species previously described. We also provide a new geographical record for N. cerasinus and the most remarkable features for each species.
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Braquiúros/genética , Código de Barras de DNA Taxonômico/métodos , Animais , DNA/genética , Larva , Filogenia , Plâncton , RNA Ribossômico 16S/genética , Especificidade da EspécieRESUMO
Gastropod shells may present large spines and sharp shapes that vary according to environmental, taxonomic, and evolutionary factors. In these cases, classic morphometric methods used to study shell contour might not provide a clear representation of morphological shell based on angular decomposition of contour. The present study analyzed and compared for the first time the robustness of the contour analysis using wavelet transformed and Elliptic Fourier descriptors for gastropod shells including enlarged spines. For that, we analyzed two geographical and ecological separated populations of Bolinus brandaris from the NW Mediterranean Sea. Results showed that contour analysis of gastropod shells with enlarged spines can be analyzed using both methodologies, but the wavelet analysis provided a better local discrimination. From an ecological perspective, shells with spines of different sizes were observed in both localities suggesting a wide plasticity of the species.
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Exoesqueleto/anatomia & histologia , Análise de Fourier , Gastrópodes/anatomia & histologia , Análise de Ondaletas , AnimaisRESUMO
OBJECTIVE: The aim of this research was to evaluate the efficacy and safety of Er:YAG lasers to improve signs and symptoms of vulvar lichen sclerosus. METHODS: The study population consisted of 28 women with symptomatic vulvar lichen sclerosus. Three nonablative, thermal-only Er:YAG laser treatment sessions (7 J/cm2, 2 Hz, 7 mm spot) were performed at 4-week intervals. Each patient was asked about the presence of symptoms, such as itching, pain, and coital pain. Clinical diagnosis was established on the basis of the presence of lesions such as hypopigmentation, ecchymosis, hyperkeratosis, excoriations, or fissures. The affected areas were divided by zones (clitoris, introitus, labia minora, labia majora, perineum, labial fusion, and effacement) and were visually evaluated. Based on the presence of symptoms, lesions, and affected zones, a scale with a maximum of 14 scores was established for use before and after treatment. The impact of lichen sclerosus on patients' lives before and after treatment was evaluated with an 11-point visual analogue scale, and treatment discomfort was assessed at each session. RESULTS: After analysis of each scoring component, individual statistically significant reductions were observed in itching, pain, ecchymosis, excoriations, and hypopigmentation. CONCLUSION: The Er:YAG laser is a safe, well tolerated, and effective method for the adjuvant treatment of vulvar lichen sclerosus. Three sessions with monthly intervals using the indicated parameters can quantifiably reduce the impact of lichen sclerosus on patients' lives.
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BACKGROUND AND OBJECTIVE: Fabry disease is an X-linked multisystemic lysosomal-storage condition. We describe a large family with a novel GLA mutation: p.M187R/g7219 T>G. PATIENTS AND METHODS: Anamnesis/physical-exam, blood/urine analysis, α-Gal-A activity and/or genetic study of at-risk individuals and multidisciplinary evaluation in confirmed cases. RESULTS: 4 males and 13 heterozygous-females displayed the mutation. Cardiac/renal/neurological disease was diagnosed at a mean age of 41/29/39 years in males and 51/56/46 years in females. Onset mean age was 20 years versus 42 years. 9/15 had cardiomyopathy. Delta wave suggestive of accessory pathway was identified in 1 male and 2 females. 1 female had cardiac arrest (ventricular fibrillation, 61 years). 2 females and 1 male died suddenly (63, 64 and 57 years). Cardiac-subscore of Mainz Severity-Score-Index was severe for males and females over 40 years. 4/15(26%) developed early renal disease. 2 males needed dialysis. 1 male died at 69 years in spite of kidney-heart transplant. CONCLUSION: We describe the largest genetically confirmed Spanish family using multidisciplinary evaluation and MSSI calculation. The novel mutation p.M187R/g7219 T>G is associated with a particularly malignant cardiac phenotype in males and females over 40 years. Severity was higher than that of the largest Spanish FOS-cohort. Short-PR with delta is being reported for the first time.
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Doença de Fabry/epidemiologia , Mutação de Sentido Incorreto , Mutação Puntual , alfa-Galactosidase/genética , Adulto , Idade de Início , Substituição de Aminoácidos , Arritmias Cardíacas/etiologia , Encéfalo/patologia , Terapia de Reposição de Enzimas , Oftalmopatias/etiologia , Doença de Fabry/complicações , Doença de Fabry/tratamento farmacológico , Doença de Fabry/genética , Doença de Fabry/patologia , Feminino , Genótipo , Parada Cardíaca/etiologia , Transplante de Coração , Humanos , Hipertrofia Ventricular Esquerda/etiologia , Rim/patologia , Doenças Renais Císticas/etiologia , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Linhagem , Espanha/epidemiologia , alfa-Galactosidase/uso terapêuticoRESUMO
OBJECTIVES: To assess the impact of a simple education program on adherence to influenza vaccination. STUDY DESIGN: Randomized open controlled study. METHODS: The education program group (EPG) received an education program via surface mail, consisting of information regarding the effectiveness and safety of the influenza vaccine. The no-program group (NPG) received no intervention. All patients in 13 primary care practices in the city of Castellón, Spain, were included if they were 60 years or older on the first day of the 2009 seasonal influenza vaccination campaign. RESULTS: There were a total of 2402 participants (mean age 70.4 [± 7.1] years); 1338 (55.7%) were women. In 2009, 950 participants (39.5%) received the vaccine, including 900 of the same participants (37.5%) who had received it in 2008 (P = .14). Of those vaccinated in 2009, 501 (52.7%) belonged to the EPG and 449 (47.3%) to the NPG (P = .01). In a logistic regression analysis we found an association between adherence to vaccination in 2009 and both vaccination in the previous year and receiving the educational intervention. Influenza-related morbidity was similar in both groups during the 7 months after vaccination. CONCLUSIONS: A mailed reminder program was effective to improve influenza vaccination rate, but only to a limited extent. Vaccination the previous year was the main predictor of adherence to vaccination. More programs are needed to improve vaccination rates in the study community.
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Educação em Saúde/organização & administração , Vacinas contra Influenza/administração & dosagem , Cooperação do Paciente/estatística & dados numéricos , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Atenção Primária à Saúde , Espanha/epidemiologiaRESUMO
Background and objective: Fabry disease is an X-linked multisystemic lysosomal-storage condition. We describe a large family with a novel GLA mutation: p.M187R/g7219 T > G. Patients and methods: Anamnesis/physical-exam, blood/urine analysis, a-Gal-A activity and/or genetic study of at-risk individuals and multidisciplinary evaluation in confirmed cases. Results: 4 males and 13 heterozygous-females displayed the mutation. Cardiac/renal/neurological disease was diagnosed at a mean age of 41/29/39 years in males and 51/56/46 years in females. Onset mean age was 20 years versus 42 years. 9/15 had cardiomyopathy. Delta wave suggestive of accessory pathway was identified in 1 male and 2 females. 1 female had cardiac arrest (ventricular fibrillation, 61 years). 2 females and 1 male died suddenly (63, 64 and 57 years). Cardiac-subscore of Mainz Severity- Score-Index was severe for males and females over 40 years. 4/15(26%) developed early renal disease. 2 males needed dialysis. 1 male died at 69 years in spite of kidney-heart transplant. Conclusion: We describe the largest genetically confirmed Spanish family using multidisciplinary evaluation and MSSI calculation. The novel mutation p.M187R/g7219 T > G is associated with a particularly malignant cardiac phenotype in males and females over 40 years. Severity was higher than that of the largest Spanish FOS-cohort. Short-PR with delta is being reported for the first time (AU)
Fundamento y objetivo: La enfermedad de Fabry es un trastorno sistémico por depósito lisosomal ligado a X. Describimos una familia grande con una mutación nueva en GLA: p.M187R/g7219 T > G. Pacientes y Métodos: Anamnesis/exploración, análisis sangre/orina), actividad alfa-Gal-A y/o estudio genético a individuos en riesgo y evaluación multidisciplinar de casos confirmados. Resultados: Cuatro varones/12 mujeres presentaban la mutación. Se diagnosticó enfermedad cardiaca/ renal/neurológica a los 41/29/39 años como media en varones, y a los 51/56/46 años en mujeres. La media de inicio fue 20 años frente a 42. Presentaban miocardiopatı' a 9/15. Se identificó onda delta indicativa de vía accesoria en un varón y 2 mujeres. Una mujer sufrió parada cardiorrespiratoria (fibrilación ventricular, 61 años). Dos mujeres y un varón murieron súbitamente (63, 64 y 57 años, respectivamente). El subscore cardiaco del Mainz Severity Score Index (MSSI) fue grave en hombres y mujeres mayores de 40 años. Presentaron enfermedad renal temprana 4/15 (26%). Dos varones precisaron diálisis. Un varón murió a los 69 años pese a trasplante cardiorrenal. Conclusión: Describimos la mayor familia española confirmada gene' ticamente con evaluación multidisciplinar y cálculo del MSSI. La nueva mutación p.M187R/g7219 T > G asocia un fenotipo cardiaco especialmente grave en hombres y mujeres mayores de 40 años. La gravedad fue superior a la de la mayor cohorte-FOS española publicada. Se describen por primera vez ondas delta asociadas a PR-corto (AU)